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Mercy CLS 6: Hematology > 14) Non-malignant granulocyte & monocyte disorders > Flashcards

14) Non-malignant granulocyte & monocyte disorders Flashcards

1
Q

monolobed (homozygous) or bilobed (heterozygous) neutrophils

A

Pelger-Huet

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2
Q

significant of Pelger Huet

A

differentiating from a left shift

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3
Q

How can monolobed Pelger Huet be distinguished from myelos?

A
  • clumped chromatin
  • mature looking cytoplasm
  • most/all neutrophils have anomaly
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4
Q

2 causes of pseudo-Pelger Huet

A
  • myeloid malignancies
  • myeloproliferative/dysplastic states
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5
Q

traits of pseudo-Pelger Huet cells

A
  • mostly monolobed
  • often hypogranular
  • <50% are hyposegmented
  • immature myeloids present too
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6
Q

inherited lysosomal storage disorder
incompletely degraded mucopolysaccharides in lysosomes

A

Alder-Reilly anomaly

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7
Q

large purple granules present in all WBCs

A

Alder-Reilly anomaly

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8
Q

Alder Reilly granules stain with ———, but toxic gran will not

A

toluidine blue

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9
Q

Alder-Reilly cells often found only in…

A

BM

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10
Q

Gasser’s cells

A
  • clusters of purple granules surrounded by clear zone
  • in lymphs
  • Alder-Reilly
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11
Q

large, grayish-blue granules
result from fusion of primary & secondary granules

A

Chediak-Higashi syndrome

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12
Q

granules are prevented from getting into lysosomes

A

Chediak-Higashi syndrome

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13
Q

Chediak-Higashi cells are often ——– and ———-; less often they are ———–

A

grans and monos

lymphs

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14
Q

defective chemotaxis, phagocytosis
↓ WBC and plt

A

Chediak-Higashi syndrome

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15
Q

often fatal in childhood due to recurrent bacterial infections

A

Chediak-Higashi syndrome

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16
Q

platelets lack large granules, function abnormally

A

Chediak-Higashi syndrome

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17
Q

physical signs of Chediak Higashi syndrome

A
  • skin hypopigmentation
  • silvery hair
  • photophobia
  • lymphadenopathy
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18
Q

grans contain inclusions that resemble Dohle bodies
made of RNA from the RER

A

May-Hegglin anomaly

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19
Q

giant platelets
mild thrombocytopenia

A

May-Hegglin anomaly

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20
Q

only symptom May-Hegglin patients may have

A

bleeding tendency

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21
Q

in lymphs, usually one big granule

A

Chediak-Higashi syndrome

22
Q

defect of mechanism to catabolize membrane lipids

A

seondary histiocytosis

23
Q

examples of hereditary lack of lysosomal enzyme needed for hydrolysis of one of the glycosidic bonds

Secondary histiocytosis

A
  • Gaucher disease
  • Neiman-Pick
  • Misc lysosomal storage diseases
24
Q

Neutrophils & macros lack microbicial activity due to defect in respiratory burt oxidase system, and cannot generate H2O2

A

chronic granulomatous disease (CGD)

25
Q

neutrophils can engulf bacteria and appear normal, but cat+ organisms can’t be killed

A

CGD

26
Q

recurrent infections with opportunistic pathogens, often life-threatening

A

CGD

27
Q

granulomas in the BM

A

CGD

28
Q

dx of CGD

A
  • nitroblue tetrazolium test (NBT)
  • flow cytometry (neutrophil oxidative burst assay)
  • cytogenetics
29
Q

explain nitroblue tetrazolium test

A
  • neutros mixed with NBT & microbes
  • normal neutros will phagocytize and generate O2 metabolites, turning NBT blue
  • if there is no resp burst, no color change
30
Q

product of CGD flow cytometry neutrophil oxidative burst assay

A

fluorescent rhodamine

31
Q

——– NBT = ———- for CGD

A

positive
negative

32
Q

2 forms of leukocyte adhesion deficiency

A

LAD I
LAD II

33
Q

↓ or absent cell surface adhesion proteins

A

LAD I

34
Q

VECs lack ligand for L-selectin, so neutrophil cannot attach to endothelium and enter tissue

A

LAD II

35
Q

dx for LAD I and II

A

flow

36
Q

Gaucher sx

A
  • pathological fracture; collapsed vertebrae; bone pain and crises; E-flask deformity; osteonecrosis; BM infiltration; osteopenia
  • hepatomegaly; splenomegaly
  • thrombocytopenia
  • anemia
37
Q

deficiency of glucocerebrosidase

A

Gaucher

38
Q

in Gaucher, the lipid ———- accumulates in the macrophages

A

glucocerebroside

39
Q

large macrophages
small eccentric nuclei
wrinkled or striated cytoplasm

A

Gaucher disease

40
Q

similar cells to Gaucher seen in…

A

rapid gran turnover (CML)

41
Q

often fatal by 3 years

A

Neimann-Pick

42
Q

2 types of Neimann-Pick

A

Type A & B

43
Q

deficiency of sphingomyelinase

A

Neimann-Pick

44
Q

in Neimann-Pick, the lipid ———– accumulates in the macrophages

A

sphingomyelin

45
Q

large macrophages
eccentric nuclei
globular cytoplasmic inclusions
“Foam cells”

A

Neimann-Pick

46
Q

Foam cells

A

Neimann-Pick

47
Q

2 types of mucopolysaccharidosis

A

Hunter’s
Hurler’s

48
Q

blue staining macrophages in BM, liver, spleen

A

sea-blue histiocytosis syndrome

49
Q

large macrophages
dense eccentric nucleus
blue or blue-green granules

A

sea-blue histiocytosis syndrome

50
Q

sea-blue histiocytosis is mostly…

A

benign

Mercy CLS 6: Hematology (25 decks)

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